CurrentCountycarePolicy 5039.CC

Evkeeza (evinacumab-dgnb) Coverage Criteria

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This policy governs prior authorization under the Health Plan medical benefit for Evkeeza in adult and pediatric patients with homozygous familial hypercholesterolemia. It applies to practitioners involved in providing the requested drug and establishes initial and annual reauthorization requirements.

Policy Summary

PayerCountycare

PolicyEvkeeza (evinacumab-dgnb) Coverage Criteria

Policy CodePolicy 5039.CC

Change TypeRevised

Effective DateJune 1, 2026

Next Review DateN/A

Key ActionSubmit prior authorization documentation showing that the patient meets all initial or reauthorization criteria, including dosing within FDA-approved manufacturer guidelines.

SourceLink

POLICY UPDATE CHANGES

A manufacturer dosing-guideline criterion was added to both the initial and reauthorization sections.

The minimum age in the initial review criteria was changed to age 1 or older.

Age 1+Minimum age for initial authorization

2 medicationsRequired LDL-lowering medications

3 monthsPraluent or Repatha trial

1 year

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CurrentCountycarePolicy 5039.CC

Evkeeza (evinacumab-dgnb) Coverage Criteria

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Policy Summary

PayerCountycare

PolicyEvkeeza (evinacumab-dgnb) Coverage Criteria

Policy CodePolicy 5039.CC

Change TypeRevised

Effective DateJune 1, 2026

Next Review DateN/A

Key ActionSubmit prior authorization documentation showing that the patient meets all initial or reauthorization criteria, including dosing within FDA-approved manufacturer guidelines.

SourceLink

POLICY UPDATE CHANGES

A manufacturer dosing-guideline criterion was added to both the initial and reauthorization sections.

The minimum age in the initial review criteria was changed to age 1 or older.

Age 1+Minimum age for initial authorization

2 medicationsRequired LDL-lowering medications

3 monthsPraluent or Repatha trial

1 year

Reauthorization duration

J1305Applicable HCPCS code

Coverage Criteria

Initial Therapy

Covered when all of the following are met:

Initial therapy

Eligibility and diagnosis
- Age: Patient is age 1 year or older.>= 1 year
- Prescriber: Evkeeza is prescribed by or in consultation with a clinical lipidologist.
- Homozygous familial hypercholesterolemia diagnosis
  - Genetic confirmation: Documented genetic testing confirms homozygous familial hypercholesterolemia.
  - Clinical scenario 1: Functional mutation(s) are documented in both LDL receptor alleles or in alleles known to affect LDL receptor functionality.
  - Clinical scenario 2: Untreated total cholesterol is greater than 500 mg/dL, triglycerides are less than 300 mg/dL, both parents have untreated total cholesterol greater than 250 mg/dL, and both parents have LDL-C greater than 500 mg/dL.TC >500 mg/dL; TG <300 mg/dL; parental untreated TC >250 mg/dL; parental LDL-C >500 mg/dL
  - Clinical scenario 3: Untreated total cholesterol is greater than 500 mg/dL, triglycerides are less than 300 mg/dL, both parents have untreated total cholesterol greater than 250 mg/dL, and either skin fibroblast LDL receptor activity is less than 20% of normal or cutaneous and tendon xanthomas with corneal arcus were present in the first decade of life.TC >500 mg/dL; TG <300 mg/dL; parental untreated TC >250 mg/dL; LDL receptor activity <20% or specified physical findings
  - Clinical scenario 4: Untreated LDL-C is greater than 500 mg/dL and either skin fibroblast LDL receptor activity is less than 20% of normal or cutaneous and tendon xanthomas with corneal arcus were present in the first decade of life.LDL-C >500 mg/dL; LDL receptor activity <20% or specified physical findings
Background LDL-lowering therapy: The patient is receiving at least two LDL-lowering medications from different classes, such as statins, nicotinic acid, or ezetimibe. One medication must be an HMG-CoA reductase inhibitor unless contraindicated or intolerant.At least 2 medications from different classes
PCSK9 inhibitor prerequisite: The patient has completed an adequate trial of at least three months of Praluent if age 18 or older or Repatha if age 10 or older, with an inadequate response, significant side effects or toxicity, or a contraindication to therapy.>=3 months
Age-specific prerequisite: Praluent age 18+; Repatha age 10+.
Baseline testing: Baseline LDL-C testing is completed within one month of initiation, and test dates are provided.Within 1 month of initiation
Reproductive potential: For females of reproductive potential, a negative pregnancy test is obtained before starting Evkeeza and effective contraception is being used.Negative pregnancy test before initiation
Applies to females of reproductive potential.
Dose: Evkeeza is prescribed within the manufacturer dosing guidelines based on the diagnosis, weight, and FDA-approved labeling.Per FDA-approved labeling

Reauthorization / Continuation Therapy

Covered for annual reauthorization when all of the following are met:

Annual continuation therapy

Initial criteria: The initial therapy criteria continue to be met, including the diagnosis, specialist involvement, required LDL-lowering therapy, prerequisite Praluent or Repatha trial, baseline and reproductive-potential requirements when applicable, and appropriate dosing.Same as initial therapy criteria
LDL response: Chart-note documentation shows a reduction in LDL levels from baseline.Reduction from baseline LDL
Dose: Evkeeza is prescribed within the manufacturer dosing guidelines based on the diagnosis, weight, and FDA-approved labeling.Per FDA-approved labeling

Coding and Clinical Thresholds

Applicable HCPCS CodeHCPCS

J1305

Injection, evinacumab-dgnb, 5mg

Minimum Age

Minimum age1 year or older

Authorization phaseRequired for initial authorization; the same initial criteria apply at reauthorization

Covered populationAdult and pediatric patients with homozygous familial hypercholesterolemia

Untreated Total Cholesterol

Untreated total cholesterolGreater than 500 mg/dL

Clinical diagnostic useRequired in the clinical HoFH scenarios that include elevated total cholesterol and triglycerides below 300 mg/dL

Measurement status

Provider Actions and Authorization Requirements

Prior Authorization

Prior Authorization Required

Evkeeza (evinacumab-dgnb) requires prior authorization under the Health Plan medical benefit. The request must satisfy all applicable initial-therapy criteria before approval.

Applies to practitioners providing the requested drug under the Health Plan’s medical benefit.
Requests that do not meet established criteria may be referred to a Medical Director for review, when required by the plan and request level.

Step Therapy

Required Praluent or Repatha Trial

Before initial Evkeeza authorization, document an adequate trial of the age-appropriate PCSK9 inhibitor with inadequate response, significant side effects/toxicity, or a contraindication.

Praluent: adequate trial of at least 3 months for members age 18 and older.
Repatha: adequate trial of at least 3 months for members age 10 and older.

Background

Evkeeza (evinacumab-dgnb) is indicated as an adjunct to diet, exercise, and other LDL-C-lowering therapies for adult and pediatric patients age 1 year and older with homozygous familial hypercholesterolemia (HoFH).

Coverage is managed through prior authorization under the Health Plan medical benefit. The policy evaluates medical necessity for this limited-indication therapy and requires documented use of other LDL-C-lowering treatments, including an adequate age-appropriate trial of Praluent or Repatha before Evkeeza is considered.

Definitions

Homozygous Familial Hypercholesterolemia (HoFH)

Genetic pathwayDocumented genetic testing confirms homozygous familial hypercholesterolemia

Functional mutation pathwayFunctional mutations are documented in both LDL receptor alleles or in alleles known to affect LDL receptor functionality

Lipid and family-history pathwaysUntreated total cholesterol greater than 500 mg/dL, triglycerides less than 300 mg/dL, and specified parental cholesterol or LDL-C thresholds are documented

LDL receptor or physical-finding pathwaysUntreated LDL-C greater than 500 mg/dL, or the applicable lipid and family-history findings, together with skin fibroblast LDL receptor activity less than 20% of normal or cutaneous and tendon xanthomas with corneal arcus in the first decade of life

Initial Therapy

The request must meet all initial review criteria:

Initial Evkeeza authorization

Age and prescriber
- Patient is age 1 year or older.>= 1 year
- Evkeeza is prescribed by or in consultation with a clinical lipidologist.
Homozygous familial hypercholesterolemia diagnosis
- Genetic confirmation: Documented genetic testing confirms homozygous familial hypercholesterolemia.

Annual Reauthorization

Annual reauthorization requires continuation of the initial criteria plus documented response:

Annual reauthorization

LDL response: Chart-note documentation shows a reduction in LDL levels from baseline.Reduction from baseline LDL
Initial criteria continue: The initial therapy criteria continue to be met, including the diagnosis, specialist involvement, required LDL-lowering therapy, prerequisite Praluent or Repatha trial, baseline and reproductive-potential requirements when applicable.Same as initial therapy criteria
Dosing:

Required Prior Therapy

Step	Prerequisite therapy	Age requirement	Trial duration	Requirement before Evkeeza
1	Praluent	Age 18 years and older	At least 3 months	Inadequate response, significant side effects or toxicity, or contraindication to therapy
1	Repatha	Age 10 years and older	At least 3 months	Inadequate response, significant side effects or toxicity, or contraindication to therapy

Policy Summary

PayerCountycare

PolicyEvkeeza (evinacumab-dgnb) Coverage Criteria

Policy CodePolicy 5039.CC

Change TypeRevised

Effective DateJune 1, 2026

Next Review DateN/A

Key ActionSubmit prior authorization documentation showing that the patient meets all initial or reauthorization criteria, including dosing within FDA-approved manufacturer guidelines.

SourceLink

On This Page

Must be untreated total cholesterol

Triglycerides

TriglyceridesLess than 300 mg/dL

Clinical diagnostic useRequired with untreated total cholesterol greater than 500 mg/dL in the applicable clinical HoFH scenarios

Measurement statusMust be untreated total cholesterol and triglyceride criteria as specified in the clinical diagnosis

Parents’ Untreated Total Cholesterol

Parents’ untreated total cholesterolGreater than 250 mg/dL in both parents

Clinical diagnostic useRequired in the applicable clinical HoFH scenarios

Measurement statusThe parental total cholesterol must be untreated

Parents’ LDL-C

Parents’ LDL-CGreater than 500 mg/dL in both parents

Clinical diagnostic useRequired in the clinical HoFH scenario that includes parental LDL-C levels

Family requirementThe threshold must be met by both parents

Skin Fibroblast LDL Receptor Activity

Skin fibroblast LDL receptor activityLess than 20% of normal

Clinical diagnostic useOne qualifying finding in specified clinical HoFH scenarios

Alternative findingCutaneous and tendon xanthomas with corneal arcus in the first decade of life may qualify instead in the applicable scenarios

Untreated LDL-C

Untreated LDL-CGreater than 500 mg/dL

Clinical diagnostic useRequired in the clinical HoFH scenario using untreated LDL-C

Additional findingThe scenario also requires skin fibroblast LDL receptor activity less than 20% of normal or the specified physical findings

Praluent or Repatha Trial

Required durationAt least 3 months

Age 18 and olderTrial of Praluent

Age 10 and olderTrial of Repatha

Baseline Testing Window

Testing windowWithin 1 month of initiation

Required documentationDates of the tests must be provided

Required baseline testingLDL-C testing; females of reproductive potential also require a negative pregnancy test before starting Evkeeza and effective contraception

Document the outcome of the trial, including inadequate response, significant side effects/toxicity, or contraindication.

Documentation Required

Diagnosis and Specialist Documentation

Document homozygous familial hypercholesterolemia (HoFH) and specialist involvement. The member must be age 1 or older and Evkeeza must be prescribed by, or in consultation with, a clinical lipidologist.

Confirm HoFH by genetic testing showing functional mutations in both LDL receptor alleles or alleles known to affect LDL receptor functionality; or document an accepted clinical diagnosis.
Accepted clinical diagnostic scenarios include the specified untreated total cholesterol, triglyceride, parental cholesterol/LDL-C, LDL receptor activity, xanthoma, corneal arcus, and untreated LDL-C findings in the policy.

Documentation Required

Baseline Laboratory Documentation

Provide baseline laboratory documentation dated within one month before Evkeeza initiation.

LDL-C result and test date are required.
For females of reproductive potential, provide a negative pregnancy test before starting Evkeeza and document use of effective contraception.

Documentation Required

Reauthorization Documentation

Annual reauthorization requires chart-note evidence that LDL levels have decreased from baseline and confirmation that Evkeeza continues to be prescribed within the manufacturer’s FDA-approved dosing guidelines.

All prior authorization renewals are reviewed annually for continued Medical Necessity.
Document the baseline LDL-C, follow-up LDL levels, and the reduction demonstrated in the chart notes.
Confirm dosing is within manufacturer guidelines based on diagnosis, weight, and other applicable factors.

Denial Risk

Failure to Meet Initial Criteria

Initial authorization may be denied if any required initial criterion is not met or cannot be documented.

Member is younger than age 1.
HoFH diagnosis is not confirmed by genetic testing or an accepted clinical diagnostic scenario.
Prescription is not by or in consultation with a clinical lipidologist.
Required LDL-lowering therapy is absent, including at least two agents from different classes and an HMG-CoA reductase inhibitor unless contraindicated or intolerant.
Required age-specific Praluent or Repatha trial is absent, inadequate, or undocumented.
Required baseline tests, pregnancy testing or contraception documentation, when applicable, are absent or not dated within one month of initiation.
Requested dose is outside manufacturer dosing guidelines.

Denial Risk

Failure to Meet Continuation Criteria

Reauthorization may be denied when annual continuation criteria are not met or the documentation does not establish ongoing Medical Necessity.

Chart notes do not show a reduction in LDL levels from baseline.
The requested dose is not within the manufacturer’s FDA-approved dosing guidelines based on diagnosis, weight, or other applicable factors.
Renewal documentation is incomplete or does not support continuation of therapy.

Functional LDL receptor mutations: Functional mutation(s) are documented in both LDL receptor alleles or in alleles known to affect LDL receptor functionality.

Lipid and family-history scenario: Untreated total cholesterol is greater than 500 mg/dL, triglycerides are less than 300 mg/dL, both parents have untreated total cholesterol greater than 250 mg/dL, and both parents have LDL-C greater than 500 mg/dL.TC >500 mg/dL; TG <300 mg/dL; parental untreated TC >250 mg/dL; parental LDL-C >500 mg/dL

Lipid, family-history, and physical-finding scenario: Untreated total cholesterol is greater than 500 mg/dL, triglycerides are less than 300 mg/dL, both parents have untreated total cholesterol greater than 250 mg/dL, and either skin fibroblast LDL receptor activity is less than 20% of normal or cutaneous and tendon xanthomas with corneal arcus were present in the first decade of life.TC >500 mg/dL; TG <300 mg/dL; parental untreated TC >250 mg/dL; LDL receptor activity <20% or specified physical findings

LDL-C and physical-finding scenario: Untreated LDL-C is greater than 500 mg/dL and either skin fibroblast LDL receptor activity is less than 20% of normal or cutaneous and tendon xanthomas with corneal arcus were present in the first decade of life.LDL-C >500 mg/dL; LDL receptor activity <20% or specified physical findings

Background LDL-lowering therapy: The patient is receiving at least two LDL-lowering medications from different classes, such as statins, nicotinic acid, or ezetimibe; one medication must be an HMG-CoA reductase inhibitor unless contraindicated or intolerant.At least 2 medications from different classes

Praluent or Repatha trial: The patient has completed an adequate trial of at least three months of Praluent if age 18 or older or Repatha if age 10 or older, with an inadequate response, significant side effects or toxicity, or a contraindication to therapy.>=3 months

Praluent applies at age 18+; Repatha applies at age 10+.

Baseline testing: Baseline LDL-C testing is completed within one month of initiation, and test dates are provided.Within 1 month of initiation

Reproductive-potential requirements: For females of reproductive potential, a negative pregnancy test is obtained before starting Evkeeza and effective contraception is being used.Negative pregnancy test before initiation

Dosing: Evkeeza is prescribed at a dose within the manufacturer dosing guidelines based on diagnosis, weight, and FDA-approved labeling.Per FDA-approved labeling

Evkeeza is prescribed at a dose within the manufacturer dosing guidelines based on diagnosis, weight, and FDA-approved labeling.

Per FDA-approved labeling